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systemic necrotizing vasculitis

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https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#1
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28386506/an-unusual-presentation-of-limited-granulomatosis-with-polyangiitis-involving-vagina-and-urethra
#2
Sandra Soro Marín, Enrique Júdez Navarro, Manuela Sianes Fernández, Ginés Sánchez Nievas, Juan Gabriel Lorenzo Romero
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28316335/complement-in-anca-associated-vasculitis-mechanisms-and-implications-for-management
#3
REVIEW
Min Chen, David R W Jayne, Ming-Hui Zhao
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of potentially life-threatening autoimmune diseases. The main histological feature in the kidneys of patients with AAV is pauci-immune necrotizing crescentic glomerulonephritis with little immunoglobulin and complement deposition in the glomerular capillary walls. The complement system was not, therefore, initially thought to be associated with the development of AAV. Accumulating evidence from animal models and clinical observations indicate, however, that activation of the complement system - and the alternative pathway in particular - is crucial for the development of AAV, and that the complement activation product C5a has a central role...
March 20, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28290145/successful-surgical-intervention-for-rectal-perforation-due-to-polyarteritis-nodosa-report-of-a-case
#4
Keiji Yoshiya, Yu Imamura, Yu Nakaji, Daisuke Taniguchi, Rinne Takeda, Koji Ando, Yuichiro Nakashima, Motohiro Shimizu, Kippei Ohgaki, Norihiro Furusyo, Takuya Matsumoto, Hiroshi Saeki, Yoshinao Oda, Eiji Oki, Yoshihiko Maehara
BACKGROUND: Polyarteritis nodosa (PAN) is a primary systemic necrotizing vasculitis with diffuse organ involvements, resulting in a high mortality rate due to multiple organ failure. Although the small bowel is the frequently targeted organ of PAN-associated vasculitis, rectal involvement is very rare, and only one case of rectal bleeding has been previously reported. The mortality rate of PAN with gastrointestinal (GI) perforation is reportedly much higher than that of without severe GI involvement...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28285286/necrotizing-polyarteritis-nodosa-like-vasculitis-in-a-child-with-systemic-lupus-erythematosus
#5
Ritambhra Nada, Joseph L Matthews, Sagar Bhattad, Anju Gupta, Surjit Singh
A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema...
February 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28210182/successful-cataract-surgery-in-a-patient-with-refractory-wegener-s-granulomatosis-effectively-treated-with-rituximab-a-case-report
#6
Abdullah M Alfawaz
Wegener's granulomatosis is a granulomatous disorder associated with systemic necrotizing vasculitis. Eye involvement occurs in approximately 50% of Wegener's granulomatosis patients and is an important cause of morbidity. Conventional treatment-related morbidity and failure have led to studies of alternative treatment modalities. In this case of a 35-year-old man with severe Wegener's granulomatosis, conventional therapy failed to induce remission. Despite the standard immunosuppressive therapy, progression of the disease was observed, mainly with ocular manifestations and renal impairment...
July 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/28208913/eosinophilic-granulomatosis-with-polyangiitis-egpa-and-hepatitis-b-infection-a-rare-association
#7
Nidhi Kaeley, Narula Kamakshi
Eosinophilic Granulomatosis with Polyangiitis (EGPA) or Churg-Strauss syndrome is a rare systemic illness which is characterized by necrotizing inflammation of small and medium sized vessels. The prominent features include asthma, eosinophilia, transient pulmonary infiltration and systemic vasculitis. Various triggering factors have been reported as putative aetiological agents of Churg-Strauss syndrome. They include infections, allergic desensitization, vaccinations like hepatitis B and influenza vaccination, cocaine abuse, drugs like leukotriene receptor antagonists and exposure to pigeons...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28166084/unexpected-complication-of-cocaine-associated-anti-neutrophil-cytoplasmic-antibody-vasculitis-related-to-persistent-in-hospital-cocaine-use
#8
Travis D Olives, Rebecca L Kornas, Russell Fujisawa, Jon B Cole
INTRODUCTION: Levamisole-adulterated cocaine has been implicated in anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. We present a case of spontaneous intraperitoneal hemorrhage, an unexpectedly severe complication of cocaine-related ANCA vasculitis, developing late during hospitalization. CASE REPORT: An adult male with a history of hepatitis C, distant cocaine use, and limited health care presented to a local emergency department (ED) with volume overload, renal failure, hyperkalemia and non-anion gap metabolic acidosis...
March 2017: Journal of Addiction Medicine
https://www.readbyqxmd.com/read/28163812/acute-urinary-retention-secondary-to-urethral-involvement-of-granulomatosis-with-polyangiitis
#9
Patrick T Anderson, Stephanie Gottheil, Manal Gabril, Lillian Barra, Nicholas Power
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period...
January 2017: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/28092221/tuberculosis-mimicking-primary-systemic-vasculitis-not-to-be-missed
#10
Ankit Jain, Durga Prasanna Misra, A Ramesh, Debdatta Basu, Vikramraj K Jain, Vir Singh Negi
Infections are an important differential diagnosis in patients presenting with features of systemic vasculitis. We report a young lady with constitutional features, leg ulcers, digital gangrene and absent peripheral pulses with cervical adenopathy. Chest imaging revealed multiple necrotic lung lesions and involvement of left subclavian artery at its origin from the aorta, Histopathology from cervical lymph nodes showed multiple caseated lymph nodes, which in the context of a positive Mantoux test led us to diagnose tuberculosis and institute appropriate therapy...
April 2017: Tropical Doctor
https://www.readbyqxmd.com/read/28079801/acute-necrotizing-retinal-vasculitis-as-onset-of-systemic-lupus-erythematosus-a-case-report
#11
Simeon Monov, Ruska Hristova, Rositza Dacheva, Reni Toncheva, Russka Shumnalieva, Viara Shoumnalieva-Ivanova, Daniela Monova
RATIONALE: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by autoantibody production, complement activation, and deposition of immune complexes in tissues and organs. SLE can involve any region of the visual system. Although ocular manifestations are not part of the classification criteria for SLE, they can be observed in up to one-third of the patients with SLE. They are rarely reported at the time of disease onset. Retinal vasculitis is usually associated with active generalized disease...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28056474/-granulomatosis-with-polyangiitis
#12
Anja Kerstein, Konstanze Holl-Ulrich, Antje Müller, Gabriela Riemekasten, Peter Lamprecht
Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA)...
January 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27955815/salmonella-meningitis-associated-with-monocyte-infiltration-in-mice
#13
Timothy J Bauler, Tregei Starr, Toni A Nagy, Sushmita Sridhar, Dana Scott, Clayton W Winkler, Olivia Steele-Mortimer, Corrella S Detweiler, Karin E Peterson
In the current study, we examined the ability of Salmonella enterica serovar Typhimurium to infect the central nervous system and cause meningitis following the natural route of infection in mice. C57BL/6J mice are extremely susceptible to systemic infection by Salmonella Typhimurium because of loss-of-function mutations in Nramp1 (SLC11A1), a phagosomal membrane protein that controls iron export from vacuoles and inhibits Salmonella growth in macrophages. Therefore, we assessed the ability of Salmonella to disseminate to the central nervous system (CNS) after oral infection in C57BL/6J mice expressing either wild-type (resistant) or mutant (susceptible) alleles of Nramp1...
January 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/27938815/acrally-distributed-dermatoses-vascular-dermatoses-purpura-and-vasculitis
#14
Jana Kazandjieva, Dimitar Antonov, Jivko Kamarashev, Nikolai Tsankov
Purpuric lesions appear in acral distribution in a variety of conditions and often provide clues to the clinical diagnosis. Purpuric means "hemorrhagic"-that is, the lesions do not blanch from pressure. This review focuses on dermatoses that produce hemorrhagic lesions in acral distribution from the large groups of the vasculitic diseases and their mimics. Cutaneous small vessel vasculitis is confined to the skin, involves mainly postcapillary venules, and has the hallmark manifestation of palpable purpura...
January 2017: Clinics in Dermatology
https://www.readbyqxmd.com/read/27919708/early-interstitial-lung-disease-in-microscopic-polyangiitis-case-report-and-literature-review
#15
Marcos García-Nava, Heidegger Mateos-Toledo, Ana Patricia Georgina Guevara-Canseco, Cesar Eduardo Infante-González, Diego Alberto Reyes-Nava, Emilio Estrada-Castro
Microscopic polyangiitis (MPA) is a systemic disease included in the Chapel Hill 2012 Classification as necrotizing vasculitis affecting capillaries, venules and arterioles. It usually expresses antineutrophil cytoplasmic antibodies (ANCA) and has a perinuclear immunofluorescence pattern and correlation with anti-myeloperoxidase (MPO) antibodies. Capillaritis with alveolar hemorrhage is the most common manifestation of lung disease. Interstitial lung disease (ILD) is uncommon, with usual interstitial pneumonia being the predominant pattern...
December 2, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27919192/predictive-value-of-neutrophil-lymphocyte-ratio-in-renal-prognosis-of-patients-with-granulomatosis-with-polyangiitis
#16
Hamit Küçük, Berna Göker, Özkan Varan, Burak Dumludag, Şeminur Haznedaroğlu, Mehmet Akif Öztürk, Abdurrahman Tufan, Tugce Emiroglu, Yasemin Erten
INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a rare necrotizing vasculitis, which usually involves the upper and lower respiratory systems and kidneys and often have a relapsing course. Neutrophil/lymphocyte ratio (NLR) has been shown to be a useful marker predicting not only progressive disease, but also mortality in various inflammatory diseases. We aimed to investigate the roles of NLR in predicting the extend of clinical involvement and prognosis of patients with GPA. MATERIALS AND METHODS: Consecutive newly diagnosed GPA patients who had follow-up for at least 6 months between 2010 and 2016 at Gazi University Internal Medicine-Rheumatology clinic were retrospectively analyzed...
December 5, 2016: Renal Failure
https://www.readbyqxmd.com/read/27917269/ecstasy-mdma-and-its-effects-on-kidneys-and-their-treatment-a-review
#17
REVIEW
Feyza Bora, Fatih Yılmaz, Taner Bora
Ecstasy (MDMA; 3,4-methylenedioxymethylamphetamine) is an illicit drug that has been increasingly abused by young people. Its effects include euphoria, enhanced sociability and heightened mental awareness. These come about via the increase of serotonin in both the central nervous system and the sympathetic nervous system. Despite the drug's prevalent abuse, serious or adverse effects are rare. Due to personal pharmacokinetics, effects from the same dosage vary according to the individual. Fatal instances may include acute hyponatremia, hyperthermia (>42 °C), disseminated intravascular coagulation (DIC) resulting from hyperthermia affecting the kidneys, and non-traumatic rhabdomyolysis...
November 2016: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/27900940/acute-kidney-injury-in-allopurinol-induced-dress-syndrome-a-case-report-of-concurrent-tubulointerstitial-nephritis-and-kidney-limited-necrotizing-vasculitis%C3%A2
#18
Anthony J Esposito, Ryan C Murphy, Mirna N Toukatly, Osama W Amro, Bryan R Kestenbaum, Behzad Najafian
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal adverse drug reaction with variable renal involvement. We report the case of a man who presented with allopurinol-induced DRESS and acute kidney injury (AKI) requiring hemodialysis. Kidney biopsy revealed eosinophilic tubulointerstitial nephritis and necrotizing vasculitis of the intralobular arteries without systemic markers of vasculitis. After cyclophosphamide and glucocorticoids, his symptoms and AKI resolved. To our knowledge, this is the first case of kidney-limited necrotizing vasculitis, questioning whether a biopsy should be routinely performed in patients with DRESS accompanied by severe AKI...
November 30, 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27893661/acute-coronary-vasospasm-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-following-nsaid-administration-a-case-report
#19
Naïl Benallegue, Pierre Lozach, Cristina Belizna, Christian Lavigne, Geoffrey Urbanski
Eosinophilic with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) is a rare systemic disease characterized by a small-vessel necrotizing vasculitis. Cardiac manifestations are broad-ranging and are associated with a poor prognosis. Coronary vasospasm is uncommon.Here, we report a case of an acute coronary vasospasm in a patient with EGPA after corticosteroids withdrawal and nonsteroidal antiinflammatory drug (NSAID) introduction. This patient was initially misdiagnosed as bradykinin-mediated angioedema...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27893578/propylthiouracil-associated-leukocytoclastic-necrotizing-cutaneous-vasculitis-a-case-report-and-review-of-the-literature
#20
Anji E Wall, Sheena M Weaver, Jeffrey S Litt, Lisa Rae
The purpose of this case report and review of the literature is to provide an exploration of the clinical symptoms, diagnosis, prevention, and management of propylthiouracil (PTU)-associated vasculitis in the intensive care setting. A PubMed search of the available literature was conducted using the MeSH search terms "propylthiouracil" and "vasculitis." The literature search returned 121 articles. Twenty-five were excluded because they were not in English. Fifty-nine case reports or case studies describing PTU-associated vasculitis were included...
May 2017: Journal of Burn Care & Research: Official Publication of the American Burn Association
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